Severe Outflow Obstruction Research Articles

Iliac vein recanalisation and stenting accelerate healing of venous leg ulcers associated with severe venous outflow obstruction.

Obstruction involving the iliac veins and/or inferior vena cava is highly comorbid in patients with chronic venous leg ulcers and is a barrier to healing. Intervention with venous stenting is recommended to promote wound healing; however, there is limited data to quantify the effects of venous outflow restoration on wound healing. We retrospectively identified patients with venous ulcers and comorbid venous outflow obstruction. Data regarding demographics, wound size, degree of obstruction, interventions, wound healing and recurrence were collected. Intervention was performed when possible and patients were grouped based on whether or not the venous outflow was reopened successfully and maintained for at least 1 year. Outcomes, including time to wound healing, wound recurrence, stent patency and ulcer-free time, were measured. Patients who maintained a patent venous outflow tract experienced higher rates of wound healing (79.3%) compared to those with persistent outflow obstruction (22.6%) at 12 months (p < 0.001). Ulcer-free time for the first year was also greater with patent venous outflow (7.6 ± 4.4months versus 1.8 ± 3.0months, p < 0.0025). Patients with severe obstruction of the venous outflow tract experience poor healing of VLUs despite appropriate wound care. Healing time is improved and ulcer-free time increased after venous intervention with stenting to eliminate obstruction.

The bladder contractility and bladder outlet obstruction indices in adult men:Results of a global Delphi consensus study.

This Delphi study was planned to examine global expert consensus with regard to utility, accuracy, and categorization of Bladder Contractility Index (BCI) and Bladder Outlet Obstruction Index (BOOI) and the related evidence. Twenty-eight experts were invited to answer the two-round survey including three foundation questions and 15 survey questions. Consensus was defined as ≥75% agreement. The ordinal scale (0-10) in round 1 was classified into "strongly agree,""agree,""neutral,""disagree," and "strongly disagree" for the final round. A systematic search for evidence was conducted for therapeutic studies that have examined outcome stratified by the indices in men. Nineteen experts participated in the survey with 100% completion. Consensus was noted with regard to 6 of 19 questions. Experts strongly agreed with utility of quantifying bladder contractility and bladder outflow obstruction with near unanimity regarding the latter. There was consensus that BCI and BOOI were accurate, that BCI was clinically useful, and for defining severe bladder outflow obstruction as BOOI > 80. Systematic search yielded 69 publications (BCI 45; BOOI 50). Most studies examined the indices as a continuous variable or by standard cutoffs (BCI 100, 150; BOOI 20, 40). There is general agreement among experts on need for indices to quantify bladder contractility and bladder outflow obstruction as well as with regard to accuracy and utility of BCI and BOOI indices. Few studies have examined the discriminant power of existing cutoffs or explored new ones. This is an extraordinary knowledge gap in the field of urology.

Minimally Invasive Surgical Resection of Accessory Mitral Valve Tissue Causing Severe Symptomatic Left Ventricular Outflow Tract Obstruction in an Adult

Objective: We report a rare case of adult onset, symptomatic, severe left ventricular outflow tract obstruction secondary to accessory mitral valve tissue that was treated surgically through a minimally invasive partial sternotomy approach. Methods: A 29-year-old female with a previous diagnosis of a sub-aortic membrane with 6 months of progressive chest pain, fatigue, palpitations, and presyncope presented to the emergency department with acute onset of severe chest pain, left arm numbness, palpitations, and light-headedness. Transthoracic and subsequent transesophageal echocardiogram demonstrated a mobile, parachute-like mass in the left ventricular outflow track with severe outflow obstruction (resting velocity 3.9m/s and peak gradient 60mmHg). The patient was transferred to our institution for surgical evaluation. Results: Review of the echocardiographic imaging demonstrated obstructing accessory mitral valve tissue (Figure A). The patient underwent surgical resection of the obstructing mass through a partial upper-sternotomy. Accessory mitral valve tissue attached to the aorto-mitral curtain was removed en bloc with chordal attachments to the posteromedial papillary muscle and muscular interventricular septum (Figure C and D). A small, hemodynamically insignificant sub-aortic membrane was also excised. There was no residual gradient, and valvular and ventricular functions were preserved (Figure B). The patient was discharged home on postoperative day 3. Conclusions: While accessory mitral valve tissue rarely presents in adulthood it should remain part of the differential diagnosis of symptomatic left ventricular outflow tract obstruction. Early surgical intervention is recommended and may be performed safely via less invasive surgical approaches. Figure. Accessory mitral valve tissue causing symptomatic, severe left ventricular outflow tract obstruction before (A) and after (B) surgical resection though a minimally invasive partial sternotomy. (C) Surgeon’s view of the left ventricular outflow tract through the aortic valve of the accessory mitral valve tissue, with severed chordae tendinae, attached to the aorto-mitral curtain beneath the left/non-coronary commissure of the aortic valve. (D) Resected accessory mitral valve tissue

Severe Outflow Obstruction After Liver Transplantation: Rescue Stapled Cavo-Cavostomy via the Right Atrial Approach and Hypothermic Circulatory Arrest.

Late vena cava/hepatic outflow obstruction after orthotopic liver transplantation (OLT), while rare, is among the most challenging complications. The cause may be an organized thrombus, reactive edema, fibrosis/scarring with calcification, or neointimal hyperplasia. Moreover, the obstruction can involve different anatomical parts of the vena cava. Surgical approaches have classically included anastomotic revision, hepatopexy, thrombectomy, cavoatrial shunt, and retransplantation. We present a case report of an innovative approach to combined inferior vena cava (IVC)/hepatic outflow obstruction in a 62-year-old female who underwent OLT using a piggyback technique for end-stage liver disease secondary to hepatitis C virus and alcohol, approximately 10 months post-transplant. A right atrial approach and hypothermic circulatory (cardiac) arrest were employed. The IVC stricture was easily resolved by performing a side to side cavo-cavostomy using an endovascular stapler (endo-GIA). In conclusion, this innovative approach is safe and effective in resolving severe outflow obstruction after liver transplantation.

Critical coarctation of the aorta: diagnostic capabilities and methods of surgical treatment of aortic coarctation in newborns

Critical coarctation of the aorta in newborns usually presents with severe narrowing of the cavotricuspid isthmus and space adjacent to the arterial duct, limiting blood flow to the thoracic aorta. Patient survival depends on duct patency. Rapid deterioration and development of multiple organ failure usually result from spontaneous closure of the patent arterial duct. This deterioration is due in part to reduced renal perfusion and results in fluid and acid retention, causing heart failure and metabolic acidosis. This may be alleviated by the administration of intravenous prostaglandin E1, with a temporary improvement in distal aortic blood flow and the overall condition of the patient. Primary repair is associated with a high risk of unfavourable outcomes due to severe systemic outflow obstruction and multiple organ failure. Therefore, as a bridge to subsequent radical surgery, palliative stenting is the preferred method in newborns with critical aortic coarctation in the decompensated state.Received 16 January 2020. Revised 29 March 2020. Accepted 30 March 2020.Funding: The study did not have sponsorship.Conflict of interest: Authors declare no conflict of interest.Author contributionsConception and design: S.M. Ivantsov, A.V. Voitov, Yu.Yu. KulyabinDrafting the article: K.A. RzaevaCritical revision of the article: I.A. Soynov, A.V. Gorbatykh, Yu.N. Gorbatykh, A.V. Bogachev-ProkophievFinal approval of the version to be published: K.A. Rzaeva, I.A. Soynov, A.V. Gorbatykh, Yu.Yu. Kulyabin, A.V. Voitov, S.M. Ivantsov, Yu.N. Gorbatykh, A.V. Bogachev-Prokophiev

Endovascular Treatment in a Case of Phelgmasia Cerulean Doleans

Background: Phlegmasia cerulean dolens (PCD) is an uncommon and severe manifestation of massive proximal venous thrombosis of the lower extremities associated with a high degree of morbidity and mortality. Case: We describe a case of a 67-year-old gentleman with metastatic gastric neuroendocrine tumour, who developed PCD of his left lower limb. He underwent endovascular thrombectomy with thrombolysis and stent placement with good effect. Discussion: Characterised by severe venous outflow obstruction, marked limb swelling, pain and discolouration, PCD can lead to venous gangrene, congestion with massive fluid sequestration and circulatory collapse if left untreated. Various treatment modalities were reported with varying outcomes, morbidity and mortality. Conclusion: A multifaceted approach to PCD may be required for successful limb salvage, taking into account the risks and benefits of each treatment modality.

Palliative stenting in a newborn with critical coarctation of the aorta

Newborns with critical coarctation of the aorta with a closed arterial duct often require surgical repair. Primary repair is associated with a high risk of unfavorable outcomes owing to severe systemic outflow obstruction and multiple organ failure. We present the case of a premature male newborn (weight = 1.9 kg) with critical coarctation of the aorta and multiple organ dysfunction syndrome with a closed arterial duct who successfully underwent isthmus stenting as a palliative step before surgical repair. The indication for primary stent implantation was decompensated metabolic acidosis. For stenting, the retroperitoneal approach was selected, and elective surgical cut-down of the right iliac artery was performed. Standard coronary stents (diameter = 4 mm; length = 13 mm) were delivered via a 4F sheath. The right iliac artery was repaired after sheath removal. The procedure was successful, and there were no procedural complications. Four days after stent implantation, the patient was stable, and he then underwent surgery. The coronary stent was extracted, the aortic isthmus was divided, and an extended anastomosis was created. The surgery was performed without complications. On the fourth day, the newborn was transferred from the intensive care unit to the ward, and on the fifteenth day, he was discharged from the hospital in good condition. Hence, palliative stenting is beneficial in newborns with critical aortic coarctation in the decompensated state because it stabilizes and prepares them for subsequent radical surgery, thereby reducing the risk of severe complications and mortality. Received 3 October 2018. Accepted 11 December 2018. Informed consent: The written informed consent has been obtained from the patient’s parents for this publication. Funding: The study did not have sponsorship. Conflict of interest: Authors declare no conflict of interest. Acknowledgment: We express our gratitude to Melina G. Galstyan for technical support in article writing.

Positive predictive value of pulse oximetry in the screening of critical congenital heart defects in term neonates

Background: Critical congenital heart defects (CCHD) are associated with poor patient outcome due to delay in diagnosis. Clinical examination alone has low positive predictive value (PPV). Pulse oximetry examination is suggested as supplemental screening tool. Aim of this study was to screen term neonates for CCHD by clinical and pulse oximetry evaluations and estimate their PPV separately and combined.Methods:Cross-sectional study of 278 term neonates weighing ≥2500g excluding those with antenatal diagnosis of congenital heart defects and/or requiring intensive care. Sample size calculated using EpiInfo version 7 taking 20,000 as population size, 7% expected frequency, 5% confidence limits and 99.9% confidence level. Ethical clearance and informed consent obtained. Clinical examination was performed within 24 hours of life followed by pre-ductal and post-ductal oxygen saturation (SpO2) recording, using standardized hand-held probe, between 24-48 hours. Neonates categorized as screen positive or negative based on an adapted algorithm. Screen positives were confirmed by echocardiography. SPSS version 16 was used for statistical analysis.Results: Mean age at clinical examination was 7.72 ± 0.32 hours during which none screened positive. Mean age at SpO2 screening was 31.93 ± 0.32 hours and a single non-syndromic, acyanotic male newborn, weighing 2550g screened positive. He was found to have severe pulmonary outflow obstruction, with ductus-dependent flow on echocardiography. PPV for pulse oximetry screening alone was 100%.Conclusions:Ductus dependent lesions missed on clinical examination may be picked up by pulse oximetry screening. Echocardiography may be used selectively to confirm diagnosis on screen positive.

Robotic trans-atrial and trans-mitral ventricular septal resection.

Localized ventricular septal hypertrophy, also known as idiopathic hypertrophic subaortic stenosis or idiopathic hypertrophic subaortic septal obstruction (IHSS), can create severe ventricular outflow obstruction. This often results in a high sub-aortic pressure gradient with potentially lethal symptoms. In 1960, Braunwald described the hemodynamic characteristics of IHSS, and thereafter, Morrow developed a trans-aortic approach to resect a large part of the ventricular septum, enlarging the outflow tract. The Morrow operation has remained the gold standard for surgically treating this pathology. However, in patients with a small aortic annulus, a severely hypertrophied septum, and a long anterior mitral leaflet, the trans-aortic approach may be more difficult, resulting in an inadequate resection and/or systolic anterior leaflet motion. The latter usually increases the obstruction and can cause mitral regurgitation. Herein, we describe a minimally invasive trans-left atrial robotic approach to treat IHSS. First, the anterior mitral leaflet is incised radially to reveal the aortic outflow tract and ventricular septum, which are located posteriorly. Thereafter, a deep "block" of septum is excised, beginning at the right valve cusp nadir and continued counterclockwise toward the left fibrous trigone. This excision is extended to the anterior papillary muscle base, where any septal connections must be divided. Subsequently, the anterior leaflet is re-suspended and the repair is completed with a flexible annuloplasty band. If necessary, the anterior leaflet can be augmented with a pericardial patch. The "wristed" robotic instruments and magnified 3-D vision definitely facilitate an adequate septal resection and anterior papillary muscle mobilization. Moreover, it is possible that this fine control helps to reduce complications, such as heart block or a ventricular septal defect.

Percutaneous Mechanical Thrombectomy Using Rotarex® S Device in Acute Limb Ischemia in Infrainguinal Occlusions.

Purpose To evaluate the effectiveness of percutaneous mechanical thrombectomy using Rotarex S in the treatment of acute limb ischemia (ALI) in infrainguinal occlusions in a retrospective study of patients treated in our institution. Methods In this study, we identified a total of 147 ALI patients that underwent mechanical thrombectomy using Rotarex S at our institution. In 82% of the cases, percutaneous thrombectomy was used as first-line treatment, and for the remainder of the cases, it was used as bailout after ineffective aspiration or thrombolysis. Additional fibrinolysis and adjunctive aspirational thrombectomy were utilized for outflow occlusion when required. Procedural outcomes, amputation rate, and mortality at 30 days were evaluated. Results Of the 147 patients treated with mechanical thrombectomy, Rotarex S was used as first-line treatment in 120 cases and as second-line treatment in 27 cases. Overall, we achieved 90.5% procedural revascularization success rate when combining mechanical thrombectomy with limited thrombolysis for severe outflow obstruction, and 1 death and 3 amputations were observed. We achieved primary success in 68.7% of the patients with the mechanical thrombectomy only, and in 21.8% of the patients, we successfully used additional limited thrombolysis in the outflow. The overall mortality was 0.7% and amputation rate was 2% at 30 days. Conclusion Percutaneous mechanical thrombectomy as first-line mini-invasive treatment in infrainguinal ALI is safe, quick, and effective, and the performance outcomes can be superior to that of traditional surgical embolectomy.

Comparison of Modified With Classic Morrow Septal Myectomy in Treating Hypertrophic Obstructive Cardiomyopathy

This study aimed to compare the efficacy and safety of the classic Morrow septal myectomy with the modified procedure in treating hypertrophic obstructive cardiomyopathy (HOCM).A retrospective study was conducted to compare the outcomes of classic with modified Morrow septal myectomy in 42 patients treated from January 2005 to July 2011. Preoperative and postoperative ventricular septal thickness, left ventricular (LV) outflow tract velocity and gradient were measured echocardiographically.In both groups, the ventricular septal thickness, LV outflow tract velocity, and LV outflow tract gradient were significantly decreased after the operation. The modified Morrow procedure group, however, showed significantly greater reduction in these echocardiographic parameters than the classic procedure group. All patients in the modified procedure group were asymptomatic postoperatively with a postoperative transvalvular pressure gradient <30 mm Hg. In the classic procedure group, only 14 (87.5%) patients, however, were asymptomatic postoperatively with a postoperative transvalvular pressure gradient <30 mm Hg, and 2 patients still had severe LV outflow obstruction postoperatively.The modified Morrow septal myectomy is safe and effective in treating HOCM patients, and is superior to the classic procedure in reducing the LV outflow tract gradient and velocity, restoring normal anatomic atrioventricular size, and alleviating symptoms associated with HOCM.

Venous angioplasty and stenting improve pelvic congestion syndrome caused by venous outflow obstruction.

Pelvic congestion syndrome (PCS) is widely thought to be due to ovarian or internal iliac vein reflux. This report of a retrospective review of treatment of nonthrombotic common iliac vein (CIV) or inferior vena cava (IVC) obstruction with relief of symptoms demonstrates an often overlooked pathologic process. Stent placement is evaluated as an effective treatment of PCS due to venous obstruction even if observed left ovarian vein (OV) reflux is left untreated. Records from two institutions were reviewed for patients with nonthrombotic venous outflow obstruction and symptoms of PCS severely affecting quality of life. The patients were evaluated with ultrasound, computed tomography (CT), and intravascular ultrasound before stent placement. From January 2008 through May 2013, 19 patients were treated with stents for severe venous outflow obstruction. Although seven patients also were found to have OV reflux, only one of these was treated with left OV coil occlusion. Whereas 10 of the 19 patients presented with a chief complaint of lower extremity pain, edema, or varicose veins, all patients described their pelvic symptoms as their dominant complaint. Ultrasound and CT suggested moderate to severe compression of the left CIV in 18 patients and a high-grade stenosis of the suprarenal IVC in one patient. Venography showed outflow obstruction with pelvic collaterals, and intravascular ultrasound confirmed focal severe stenosis of theinvolved vein. Follow-up of 1 to 59months (median, 11months) revealed complete resolution of pelvic pain in 15 of 19 patients and of dyspareunia in 14 of 17 sexually active patients. Of the 15 patients who experienced left lower extremity pain or edema before treatment, 13 experienced complete resolution after treatment. Imaging follow-up byultrasound or CT showed 16 of the stents to be widely patent, with 3 minor asymptomatic stenoses. Nonthrombotic obstruction of the left CIV or IVC is an underappreciated cause of PCS. Venous angioplasty and stenting provide excellent short-term results for such patients, with resolution of chronic pelvic pain and dyspareunia. Venous obstruction should be considered andcarefully evaluated in patients presenting with pelvic congestion, and treatment of obstruction alone may solve the patient's symptoms.

English

Phlegmasia cerulean dolens is a severe form of deep venous thrombosis characterized by severe venous outflow obstruction, marked limb swelling, pain, bluish discoloration, and even venous gangrene, if the condition is untreated. Etiological factors include malignancy, femoral vein catheterization, heparin – induced thrombocytopenia, anti phospholipid syndrome, surgery, heart failure, and pregnancy.

Exercise Hemodynamics and Risk Assessment in Asymptomatic Aortic Stenosis

More than half a century ago, in the American Journal of Cardiology , Dr Paul Wood aptly described aortic stenosis (AS) as “a simple mechanical fault which, if severe enough, imposes a heavy burden on the left ventricle and sooner or later overcomes it.”1 Our subsequent understanding of the pathophysiology of AS, using more sophisticated tools than those at Dr Wood's disposal, has confirmed his concept. Severe chronic pressure overload and outflow obstruction beget left ventricular (LV) hypertrophy and its attendant myocardial fibrosis and impaired coronary vasodilator reserve, leading to LV systolic and diastolic dysfunction, and progressing ultimately to the anticipated symptoms of dyspnea, angina, and presyncope or syncope, which are harbingers of death within a few years. Once symptoms develop, the treatment strategy is clear, because aortic valve replacement (AVR) improves symptoms, improves LV function, and improves survival. The indications for AVR in asymptomatic patients, however, remain less clear and the subject of ongoing debate.2–4 Although the current evidence-based guidelines recommend a watchful waiting approach for most patients, with no class I indications for surgery in asymptomatic patients with normal LV systolic function,5,6 numerous studies have shown that patients with severe AS have a high likelihood of developing symptoms and requiring surgery within 3 to 5 years,7–10 and some series have reported that asymptomatic patients with severe AS are also at risk of death when managed without surgery.11,12 Because the operative risk of AVR is low in experienced centers,11,13 there is intense interest in identifying the subsets of asymptomatic patients that have the highest likelihood of dying or developing symptoms over the short term and that might benefit from early, preemptive AVR rather than a watchful waiting approach. Article see p 851 Determining …

Congenital giant cardiac tumor with severe left-ventricular inflow and outflow obstruction and arrhythmia treated with pulmonary artery banding and long-term amiodarone infusion

We report a congenital giant cardiac tumor that occupied the majority of left ventricular cavity with severe left ventricular inflow and outflow obstruction. The hemodynamics were similar to univentricular physiology. He was treated with prostaglandins and bilateral pulmonary artery banding. He had frequent supraventricular tachycardia associated with ventricular pre-excitation that was controlled by long-term administration of intravenous amiodarone. The patient died due to sepsis after 3 months.

Acute Budd Chiari and Underlying JAK 2 Mutation Requiring TIPS Placement

Purpose: The JAK2V617F mutation is strongly implicated in the pathogenesis of myeloproliferative disorders (MPD) which account for at least 50% of cases of Budd-Chiari Syndrome (BCS). MPD is a common cause of splanchnic vein thrombosis in young women. Patients with BCS and underlying JAK2 mutation have severe hepatic outflow obstruction with worse Child-Pugh scores, and often require decompressive procedures such as transjugular intrahepatic portosystemic shunts (TIPS) placement or liver transplantation compared to those without the mutation. A 19 year old Caucasian woman with chronic abdominal pain, nausea and vomiting was found to have splenic vein thrombus on ultrasound. An initial hypercoagulable workup was negative. She was instructed to discontinue her oral contraceptives and started on anticoagulation with warfarin. Imaging studies two months later showed resolution of the thrombus. She was noncompliant with her anticoagulation and presented six months later with similar symptoms. A repeat ultrasound of the abdomen showed thrombosis of two of her hepatic veins, right portal vein and splenic vein. She was immediately restarted on anticoagulation. She was noted to have a mildly elevated ALT in the 50-60 range but her other liver function tests remained normal. She also had a normal CBC except for mild anemia and a normal INR. Further workup revealed a positive JAK2 mutation. She was admitted one month later with worsening abdominal pain, new large ascites and occlusion of all three hepatic veins despite anticoagulation. She was diagnosed with acute BCS. Hepatic venography demonstrated “spider web” collaterals of the right and left hepatic veins consistent with BCS. She underwent a large volume paracentesis and successful TIPS placement with decrease in her hepatic venous pressure gradient from 27 to 5 mm Hg. Liver biopsy showed evidence of moderate to severe sinusoidal dilation in zone 3 consistent with BCS. She recovered well with resolution of her ascites and abdominal pain. She was discharged home with the anticipation of lifelong anticoagulation. Patients with newly diagnosed BCS should be routinely screened for JAK2 mutations. Unlike the majority of patients with MPD, this subgroup of patients have normal hemoglobin levels, platelet counts and erythropoietin levels making diagnosis difficult with laboratory values alone. These patients present with severe hepatic outflow obstruction which portends a worse clinical outcome, and thus should be aggressively intervened upon at time of presentation to ensure long-term survival.

Contraindications to percutaneous tracheostomy due to anomaly of aortic-arch branches origin and running: relative or absolute

initial repair, we obtained a competent valve and also severe outflow obstruction due to systolic anterior motion (SAM) that we unsuccessfully tried to address with a conventional A2–P2 Alfieri repair. SAM persisted; hence, we removed the midline A2–P2 Alfieri stitch and placed a new edge-to-edge stitch now between the A1–P1 segments as shown in the picture. Actually, in the picture, we can see the most anterolateral of the two sets of GoreTex neochordae (in the A2 scallop close to A1) used to correct the prolapse of A2. We can also see the green pledgeted Ti-Cron sutures used for the posterior annulus plication after the quadrangular resection that mark the midline of the mitral valve. With these two references, it is clear that the newly placed Alfieri stitch was moved, as explained in the text, towards the anterolateral commissure and now holds the A1–P1 scallops together. We hope this helps clarify this technique that allowed us to preserve the mitral valve in this case of complex repair for myxomatous bileaflet prolapse.

PHLEGMASIA CERULEA DOLENCE- RISK FACTORS AND PREVENTION. /CASE REPORT/

Phlegmasia cerulean dolens is a severe form of deep venous thrombosis characterized by severe venous outflow obstruction, marked limb swelling, pain, bluish discoloration, and even venous gangrene, if the condition is untreated. Etiological factors include malignancy, femoral vein catheterization, heparin-induced thrombocytopenia, antiphospholipid syndrome, surgery, heart failure, and pregnancy. In our case, 71 year-old woman, with twenty three years treated diabetes mellitus was accepted for GI imaging study for suspicions of neoplastic process of stomach. On the third day she complained of edema, agonizing pain and cyanosis, many blisters and starting necrosis on the dorsal and lateral surface of right ankle, progressively extended to distal one third of leg. Anticoagulation with intravenous administration of heparin, and fluid resuscitation started immediately.

Bidirectional Glenn Shunt as an Adjunct to Surgical Repair of Congenital Heart Disease Associated with Pulmonary Outflow Obstruction: Relevance of the Fluid Pressure Drop–Flow Relationship

A bidirectional Glenn shunt (BGS) was successfully incorporated into a two-ventricle repair for 10 patients (age, 3-17 years) who had congenital heart disease associated with severe pulmonary outflow obstruction. The BGS was used to volume-unload the pulmonary ventricle faced with residual outflow obstruction, thereby avoiding the need for insertion of a ventricle-to-pulmonary artery conduit. Transthoracic Doppler flow velocity analysis was used to determine transpulmonary peak systolic pressure drops as a measure of obstruction. Preoperative values ranged from 70 to 100 mmHg, and postoperative values ranged from less than 10 to 16 mmHg. At this writing, all patients are doing well 15 to 52 months after surgery. To gain further insight into the reduced pressure drop that may be achieved by decreasing flow rate across obstruction, a computer-based description of fluid flow was used to simulate blood traversing circumferentially narrowed passages. Overall pressure drops and associated flow energy losses were determined from numeric solutions (using finite-element analysis) to the Navier-Stokes equations for the proposed fluid reactions. Pressure drops and flow energy losses were found to decrease dramatically as flow rate was progressively reduced. For selected patients, a BGS can be an effective adjunct to the surgical treatment of pulmonary outflow obstruction. This approach avoids the use of a ventricle-to-pulmonary artery conduit, and thus the inevitable need in most patients for reoperations because of somatic growth, conduit failure, or both.

Oral Disopyramide for the Acute Treatment of Severe Outflow Obstruction in Hypertrophic Cardiomyopathy in the ICU Setting

When severe COPD and obstructive hypertrophic cardiomyopathy (HCM) coexist, management is challenging and complex. Drug contraindications limit pharmacologic options. Patients may not be candidates for surgical septal myectomy due to severe pulmonary disease. We describe a case of an elderly woman with severe reactive COPD who presented with an infectious exacerbation and dyspnea that progressed to near intubation due to heart failure from coexistent obstructive HCM. Transthoracic echocardiography revealed massive asymmetric septal hypertrophy and a diffusely hyperkinetic left ventricle with a left ventricular outflow tract (LVOT) gradient of 92 mm Hg. Two and a half hours after oral administration of disopyramide, LVOT gradient had decreased to 25 mm Hg with a corresponding immediate improvement in symptoms.